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囊性部分分化型肾母细胞瘤:婴儿肾肿瘤谱系中的一种实体。

Cystic partially differentiated nephroblastoma: an entity in the spectrum of infantile renal neoplasia.

作者信息

Joshi V V

出版信息

Perspect Pediatr Pathol. 1979;5:217-35.

PMID:231757
Abstract

Clinicopathologic features of 17 cases of "cystic partially differentiated nephroblastoma" (CPDN) including three seen in personal experience have been reviewed. CPDN has previously been designated by various terms such as "benign multilocular cystic nephroma," "polycystic nephroblastoma," and "cystic nephroma." The term CPDN is preferred because it aptly denotes the characteristic gross and microscopic features and the probable histogenesis of the lesion. CPDN occurs before 2 years of age. Clinically, abdominal mass is the most common presenting feature. CPDN is often diagnosed as Wilms' tumor on clinical and radiologic grounds. Grossly, the lesion is almost totally or predominantly multicystic. Microscopically, the cysts are usually lined by flattened to cuboidal epithelium. The septa contain a mixture of blastemal cells and poorly differentiated and well-differentiated epithelial and mesenchymal elements. It is this feature which distinguishes CPDN from multilocular cyst of the kidney. Although there is a suggestion that there might be two subtypes of CPDN-one with predominantly well-differentiated elements and the other with predominantly poorly differentiated elements-no definite conclusions can be drawn regarding the existence of such subtypes on the basis of available data. Arguments could be advanced in favor of both the neoplastic and dysgenetic nature of CPDN. However, the view that CPDN is a neoplastic lesion is favored. On the basis of available data, CPDN appears to take a benign course, and simple nephrectomy seems to be the treatment of choice. However, in view of the possibility of recurrence as shown by rare cases of congenital mesoblastic nephroma (another usually benign lesion in the spectrum of infantile renal necoplasia), regular follow-up is recommended.

摘要

对17例“囊性部分分化型肾母细胞瘤”(CPDN)的临床病理特征进行了回顾,其中3例是本人经验所见。CPDN此前曾有多种命名,如“良性多房性肾囊肿”“多囊性肾母细胞瘤”和“肾囊肿”。首选CPDN这个术语,因为它恰当地描述了该病变的特征性大体和显微镜下特征以及可能的组织发生。CPDN发生于2岁之前。临床上,腹部肿块是最常见的表现特征。CPDN在临床和影像学上常被诊断为肾母细胞瘤。大体上,病变几乎完全或主要为多囊性。显微镜下,囊肿通常内衬扁平至立方形上皮。间隔包含胚芽细胞以及分化差和分化良好的上皮和间充质成分的混合物。正是这一特征将CPDN与肾多房囊肿区分开来。尽管有人提出CPDN可能有两种亚型——一种主要为分化良好的成分,另一种主要为分化差的成分——但根据现有数据,无法就这些亚型的存在得出明确结论。关于CPDN的肿瘤性和发育异常性质都有论据支持。然而,CPDN是肿瘤性病变的观点更受青睐。根据现有数据,CPDN似乎呈良性病程,单纯肾切除术似乎是首选治疗方法。然而,鉴于先天性中胚叶肾瘤(婴儿肾肿瘤谱系中的另一种通常为良性的病变)的罕见病例显示有复发的可能性,建议定期随访。

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